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We herein report the histopathological findings of the temporal bone taken from a patient with unilateral profound deafness since early childhood. The patient was a 72-year-old male who died of lung cancer and extensive metastases including the tongue. The patient had a history of profound hearing loss in his left ear since childhood. The histopathological finding of the left temporal bone revealed a severe atrophy of the organ of Corti, a detached and rolled-up tectorial membrane, a moderate loss of the stria vascularis, and a severe loss of spiral ganglion cells. In addition, the macula of the saccule was severely degenerated. The marked degeneration in the inner ear indicated a cochleosaccular disorder, which is a typical temporal bone finding in cases of viral labyrinthitis and hereditary hearing impairment. The present patient was suspected to have suffered cochleosaccular degeneration as a result of an inner ear viral infection during childhood because the number of spiral ganglion cells was significantly reduced because of secondary neural degeneration.

Temporal Bone Histopathology Exhibiting Cochleosaccular Degeneration in a Patient with Profound Deafness